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Naphthol+AS-BI-phosphat+Dinatriumsalz
Artikel-Nr:
(BOSSBS-12462R-FITC)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-12462R-FITC
Lokale Artikelnummer::
BOSSBS-12462R-FITC
Beschreibung:
AGPS is a 658 amino acid enzyme that is required for glycerolipid metabolism and ether lipid biosynthesis. Localized to the inner aspect of the peroxisomal membrane, AGPS is likely part of a heterotrimeric complex that is also composed of GNPAT and a modified form of GNPAT. Containing one FAD-binding PCMH-type domain, AGPS utilizes FAD as a cofactor in the synthesis of alkyl-glycerone 3-phophate and a long-chain acid anion from 1-acteyl-glyerone 3-phosphate and a long-chain alcohol. Defects in the gene encoding AGPS results in rhizomelic chondrodysplasia punctata type 3, a disease characterized by vertebral disorders, severe mental retardation, cutaneous lesions, cataracts and rhizomelic shortening of the humerus and femur.
VE:
1 * 100 µl
Artikel-Nr:
(78268-1ML-F)
Lieferant:
Sigma-Aldrich
Hersteller-Artikelnummer::
78268-1ML-F
Lokale Artikelnummer::
SUPL78268-1ML-F
Beschreibung:
N-Methyl-bis-heptafluorobutyramide (MBHFBA) ≥96,0% (durch GC), LiChropur™ zur GC-Derivatisierung, Supelco®
VE:
1 * 1 mL
Artikel-Nr:
(BOSSBS-10460R)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-10460R
Lokale Artikelnummer::
BOSSBS-10460R
Beschreibung:
This gene encodes two of three subunit types of the membrane-bound enzyme N-acetylglucosamine-1-phosphotransferase, a heterohexameric complex composed of two alpha, two beta, and two gamma subunits. The encoded protein is proteolytically cleaved at the Lys928-Asp929 bond to yield mature alpha and beta polypeptides while the gamma subunits are the product of a distinct gene (GeneID 84572). In the Golgi apparatus, the heterohexameric complex catalyzes the first step in the synthesis of mannose 6-phosphate recognition markers on certain oligosaccharides of newly synthesized lysosomal enzymes. These recognition markers are essential for appropriate trafficking of lysosomal enzymes. Mutations in this gene have been associated with both mucolipidosis II and mucolipidosis IIIA.[provided by RefSeq, May 2010].
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-11696R-A350)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-11696R-A350
Lokale Artikelnummer::
BOSSBS-11696R-A350
Beschreibung:
The Huntingtin protein contains a polyglutamine region, which leads to Huntingtin’s disease (HD) when the number of glutamine repeats exceeds thirty-five. The mutated Huntingtin protein acts within the nucleus to induce neurodegeneration by a cell-specific apoptotic mechanism. The loss of activity of the Huntingtin protein may be contributed to abnormal interactions between the mutant protein and other associated cellular proteins. Huntingtin interacts with a variety of proteins including HAP1, glyceraldehyde phosphate dehydrogenase (GAPDH), HAP40, Rab5 and HIP1. HAP40 mediates the recruitment of Huntingtin by Rab5 onto early endosomes. Specifically, this complex regulates endosome motility, which may be a key event of the pathogenetic process leading to neurodegeneration in HD.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-11708R-A647)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-11708R-A647
Lokale Artikelnummer::
BOSSBS-11708R-A647
Beschreibung:
ATP13A2 is a 1,180 amino acid multi-pass membrane protein that belongs to the P5 subfamily of ATPases which play an important role in the transportation of inorganic cations. Expressed as multiple alternative spliced isoforms, ATP13A2 functions to catalyze the conversion of ATP to ADP and a free phosphate, thereby participating in the active transport of ions across cellular membranes. Defects in the gene encoding ATP13A2 are the cause of Kufor-Rakeb syndrome (KRS), a rare hereditary type of Parkinson’s disease that exhibits juvenile onset and is characterized by neurodegeneration and dementia. The ATP13A2 gene maps to human chromosome 1, which spans 260 million base pairs, contains over 3,000 genes and comprises nearly 8% of the human genome.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-13507R-FITC)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-13507R-FITC
Lokale Artikelnummer::
BOSSBS-13507R-FITC
Beschreibung:
G protein-coupled receptors (GPCRs), also designated seven transmembrane (7TM) receptors and heptahelical receptors, are a protein family which interact with G proteins (heterotrimeric GTPases) to synthesize intracellular second messengers such as diacylglycerol, cyclic AMP, inositol phosphates, and calcium ions. Their diverse biological functions range from vision and olfaction to neuronal and endocrine signaling and are involved in many pathological conditions. G protein receptor 84 (GPR84), a member of the GCPR 1 family, is an orphan GCPR expressed in bone marrow, brain, heart, muscle, colon, thymus, spleen, kidney, liver, placenta, intestine, lung and peripheral blood leukocytes. In activated T cells, GPR84 regulates early interleukin-4 (IL-4) gene expression.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-9667R-A350)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-9667R-A350
Lokale Artikelnummer::
BOSSBS-9667R-A350
Beschreibung:
DENND2C is a 928 amino acid protein that contains a dDENN domain, a DENN domain, and a uDENN domain and exists as three isoforms as a result of alternative splicing. The DENND2C protein is thought to target to actin filaments and control Rab9-dependent trafficking of mannose-6-phosphate receptor to lysosomes. The gene encoding DENND2C maps to human chromosome 1, the largest human chromosome which spans about 260 million base pairs and makes up 8% of the human genome. Other notable genes located on chromosome 1 include LMNA, which is associated with the rare aging disease Hutchinson-Gilford progeria, and the MUTYH gene, which is partially responsible for familial adenomatous polyposis. Stickler syndrome, Parkinsons, Gaucher disease and Usher syndrome.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-9667R-HRP)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-9667R-HRP
Lokale Artikelnummer::
BOSSBS-9667R-HRP
Beschreibung:
DENND2C is a 928 amino acid protein that contains a dDENN domain, a DENN domain, and a uDENN domain and exists as three isoforms as a result of alternative splicing. The DENND2C protein is thought to target to actin filaments and control Rab9-dependent trafficking of mannose-6-phosphate receptor to lysosomes. The gene encoding DENND2C maps to human chromosome 1, the largest human chromosome which spans about 260 million base pairs and makes up 8% of the human genome. Other notable genes located on chromosome 1 include LMNA, which is associated with the rare aging disease Hutchinson-Gilford progeria, and the MUTYH gene, which is partially responsible for familial adenomatous polyposis. Stickler syndrome, Parkinsons, Gaucher disease and Usher syndrome.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-13366R-FITC)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-13366R-FITC
Lokale Artikelnummer::
BOSSBS-13366R-FITC
Beschreibung:
GK2 is a 553 amino acid protein that belongs to the FGGY kinase family and is involved in the pathway of glycerol degradation. Localized to the outer membrane of the mitochondrion and expressed at high levels in testis, GK2 functions to catalyze the ATP-dependent conversion of glycerol to glycerol 3-phosphate. Via its catalytic activity, GK2 plays an essential role in the regulation of glycerol uptake and metabolism. The gene encoding GK2 maps to chromosome 4, which encodes nearly 6% of the human genome and has the largest gene deserts (regions of the genome with no protein encoding genes) of all of the human chromosomes. Defects in some of the genes located on chromosome 4 are associated with Huntington's disease, Ellis-van Creveld syndrome, methylmalonic acidemia and polycystic kidney disease.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-9035R-FITC)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-9035R-FITC
Lokale Artikelnummer::
BOSSBS-9035R-FITC
Beschreibung:
Pyridoxal kinase belongs to the pyridoxine kinase family and phosphorylates vitamin B6, a step necessary for the conversion of vitamin B6 to pyridoxal 5’ phosphate (PLP), the active form of vitamin B6. PLP acts as a coenzyme and functions to maintain homeostasis. Pyridoxal kinase is a 312-amino acid cytoplasmic protein that may act as a homodimer and is expressed ubiquitously. There are three known isoforms of pyridoxal kinase, and isoform 3 expression is observed in adult testis and spermatozoa. The optimum pH for pyridoxal kinase is between 5.5 and 6.0. PDXK, the gene that encodes the pyridoxal kinase protein, maps to chromosome 21q22.3 and may be a candidate gene for autoimmune polyglandular disease type 1, a genetic disorder that has been mapped to the same region on chromosome 21.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-13267R-HRP)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-13267R-HRP
Lokale Artikelnummer::
BOSSBS-13267R-HRP
Beschreibung:
GALK2 is important in the first step of the galactose metabolism pathway. GALK1, which belongs to the GHMP kinase family of proteins, is a crucial enzyme for galactose metabolism, specifically converting ?d-galactose to galactose 1-phosphate. Defects in the gene encoding GALK1 can cause galactosemia II, an autosomal recessive disorder characterized by congenital cataracts during infancy, often within the first two weeks of life. In the adult population, galactosemia II can cause presenile cataracts that are secondary to accumulation of galactitol in the lens of the eye. A second gene, GALK2, encodes an enzyme with greater activity against GalNAc than galactose. GALK2 has been implicated in the salvage pathway for the reutilization of free GalNAc derived from the degradation of complex carbohydrates.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBSM-0855M-A750)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BSM-0855M-A750
Lokale Artikelnummer::
BOSSBSM-0855M-A750
Beschreibung:
Insulin is a pancreatic hormone that regulates glucose and is involved in the synthesis of protein and fat. It increases cell permeability to monosaccharides, amino acids and fatty acids. It accelerates glycolysis, the pentose phosphate cycle, and glycogen synthesis in liver. Heterodimer of a B chain and an A chain linked by two disulfide bonds.Belongs to the insulin family. The insulin-link growth factors, IGF-I and IGF-II (also desinated somatomedin C and multiplication stimulating activator, respectvely), share approximatly 76% sequence identity and are 50% related to pro-insulin.IGF-I and IGF-II are nonglycosylated, single chain proteins of 70 and 76 amino acids in length, respectivelly. IGF-I functions as an autocrine regulator of growth in vaious, whereas the function of IGF-II is less well defined.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-11217R-A555)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-11217R-A555
Lokale Artikelnummer::
BOSSBS-11217R-A555
Beschreibung:
Myotubularin-related protein 14 (MTMR14), also known as Jumpy, is a myotubularin-related phosphoinositol-3-phosphate (PI3P) phosphatase (1). Mutations in the MTMR14 gene have been associated with centronuclear myopathy (1). MTMR14 deficiency in mice leads to altered calcium homeostasis and muscle disorders (2). MTMR14 has also been shown to play a role in autophagy, a process that is highly regulated by phosphatidylinositides through the type III PI3K, Vps34 (3). MTMR14 was localized to autophagic isolation membranes and early autophagosomes (3). In these studies, MTMR14 inhibited autophagy and mutations of MTMR14 associated with centronuclear myopathy were also defective in autophagy inhibition. In zebrafish, MTMR14 knockdown was shown to increase the number of autophagosomes, suggesting that its activity is associated with an inhibition of autophagy (4).
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-13366R-A488)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-13366R-A488
Lokale Artikelnummer::
BOSSBS-13366R-A488
Beschreibung:
GK2 is a 553 amino acid protein that belongs to the FGGY kinase family and is involved in the pathway of glycerol degradation. Localized to the outer membrane of the mitochondrion and expressed at high levels in testis, GK2 functions to catalyze the ATP-dependent conversion of glycerol to glycerol 3-phosphate. Via its catalytic activity, GK2 plays an essential role in the regulation of glycerol uptake and metabolism. The gene encoding GK2 maps to chromosome 4, which encodes nearly 6% of the human genome and has the largest gene deserts (regions of the genome with no protein encoding genes) of all of the human chromosomes. Defects in some of the genes located on chromosome 4 are associated with Huntington's disease, Ellis-van Creveld syndrome, methylmalonic acidemia and polycystic kidney disease.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-9035R-CY3)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-9035R-CY3
Lokale Artikelnummer::
BOSSBS-9035R-CY3
Beschreibung:
Pyridoxal kinase belongs to the pyridoxine kinase family and phosphorylates vitamin B6, a step necessary for the conversion of vitamin B6 to pyridoxal 5’ phosphate (PLP), the active form of vitamin B6. PLP acts as a coenzyme and functions to maintain homeostasis. Pyridoxal kinase is a 312-amino acid cytoplasmic protein that may act as a homodimer and is expressed ubiquitously. There are three known isoforms of pyridoxal kinase, and isoform 3 expression is observed in adult testis and spermatozoa. The optimum pH for pyridoxal kinase is between 5.5 and 6.0. PDXK, the gene that encodes the pyridoxal kinase protein, maps to chromosome 21q22.3 and may be a candidate gene for autoimmune polyglandular disease type 1, a genetic disorder that has been mapped to the same region on chromosome 21.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-9035R-A350)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-9035R-A350
Lokale Artikelnummer::
BOSSBS-9035R-A350
Beschreibung:
Pyridoxal kinase belongs to the pyridoxine kinase family and phosphorylates vitamin B6, a step necessary for the conversion of vitamin B6 to pyridoxal 5’ phosphate (PLP), the active form of vitamin B6. PLP acts as a coenzyme and functions to maintain homeostasis. Pyridoxal kinase is a 312-amino acid cytoplasmic protein that may act as a homodimer and is expressed ubiquitously. There are three known isoforms of pyridoxal kinase, and isoform 3 expression is observed in adult testis and spermatozoa. The optimum pH for pyridoxal kinase is between 5.5 and 6.0. PDXK, the gene that encodes the pyridoxal kinase protein, maps to chromosome 21q22.3 and may be a candidate gene for autoimmune polyglandular disease type 1, a genetic disorder that has been mapped to the same region on chromosome 21.
VE:
1 * 100 µl
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 noch angezeigt wird und Sie Hilfe benötigen, rufen Sie uns an 1-800-932 - 5000.
Lager für diesen Artikel ist begrenzt, kann aber in einem Lagerhaus in Ihrer Nähe zur Verfügung. Bitte stellen Sie sicher, dass Sie in sind angemeldet auf dieser Seite, so dass verfügbare Bestand angezeigt werden können. Wenn das
noch angezeigt wird und Sie Hilfe benötigen, rufen Sie uns an 1-800-932 - 5000.
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